AJR 2001; 176:953-954
© American Roentgen Ray Society
Wide-Mouthed Sacculations in the Esophagus
A Radiographic Finding in Scleroderma
Claire A. Coggins1,
Marc S. Levine1,
Craig D. Kesack2 and
David A. Katzka3
1
Department of Radiology, Hospital of the University of Pennsylvania, 3400
Spruce St., Philadelphia, PA 19104.
2
Department of Radiology, Doylestown Hospital, 595 W. State St., Doylestown, PA
18901.
3
Department of Medicine, Hospital of the University of Pennsylvania,
Philadelphia, PA 19104.
Received July 31, 2000;
accepted after revision September 22, 2000.
Address correspondence to M. S. Levine.
Introduction
Scleroderma is a multisystem disorder of small vessels and connective
tissue that involves the gastrointestinal tract in up to 90% of patients
[1]. The most common site of
gastrointestinal involvement is the esophagus, followed by the anorectal
region, small bowel, and colon
[1]. Scleroderma predominantly
affects the smooth muscle layer of the bowel wall, causing atrophy and
fragmentation of smooth muscle, followed by collagen deposition and fibrosis.
In the esophagus, this condition may be manifested by severe esophageal
dysmotility with absent primary peristalsis in the smooth muscle portion of
the esophagus, a patulous gastroesophageal junction with massive
gastroesophageal reflux, reflux esophagitis, peptic strictures, Barrett's
esophagus, and even esophageal adenocarcinomas
[2,3,4].
To our knowledge, however, wide-mouthed sacculations in the esophagus similar
to those in the small bowel or colon have not been described previously as a
radiographic manifestation of scleroderma involving the esophagus. We,
therefore, report an unusual case of wide-mouthed sacculations in the
esophagus in a patient with scleroderma.
Case Report
A 65-year-old woman presented with Raynaud's phenomenon, severe substernal
burning, dyspnea on exertion, and postprandial coughing. Physical examination
revealed a marked decrease in oral aperture, skin tightening, and bilateral
diffuse rales on auscultation. A double-contrast upper gastrointestinal
examination revealed a moderately dilated flaccid esophagus with absent
primary peristalsis below the thoracic inlet, a patulous gastroesophageal
junction with multiple episodes of gastroesophageal reflux as far proximally
as the thoracic inlet, and a nodular mucosa in the distal esophagus compatible
with reflux esophagitis. Two wide-mouthed sacculations or diverticula were
also seen in the esophagus: one was in the mid thoracic esophagus at the level
of the carina and the other was in the upper thoracic esophagus just above the
level of the aortic arch (Fig.
1A,1B).
The sacculations had diameters of 4 cm and 3 cm, respectively. Interstitial
disease was also noted at both lung bases. A nuclear scintigram with
radiolabeled water revealed prolonged accumulation of radioactivity in the
lower end of the thoracic esophagus, most likely resulting from a combination
of slow emptying and gastroesophageal reflux. The scan also revealed two areas
of persistent activity in the upper thoracic esophagus, presumably within
these wide-mouthed diverticula. Esophageal manometry revealed absent
peristaltic contractions in the thoracic esophagus as far proximally as the
thoracic inlet. The findings on all these examinations were attributed to
esophageal involvement by scleroderma. Subsequent blood tests revealed
hematologic findings of scleroderma, including a positive antinuclear antibody
with a speckled pattern and a titer of 1:2560. The diagnosis was also
confirmed by consultation with a rheumatologist. The patient had marked
improvement of her reflux symptoms after treatment with high-dose proton pump
inhibitors.
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Fig. 1A. —Wide-mouthed sacculations in esophagus in 65-year-old woman
with scleroderma. Upright left posterior oblique spot image from
double-contrast esophagography shows two wide-mouthed sacculations en face
(black arrows) in upper and mid thoracic esophagus. Note how upper
sacculation extends superiorly just above level of aortic arch (white
arrow).
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Fig. 1B. —Wide-mouthed sacculations in esophagus in 65-year-old woman
with scleroderma. Upright right posterior oblique spot image from
double-contrast esophagography shows sacculations in profile
(arrows).
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Discussion
Wide-mouthed sacculations or diverticula have been well documented in both
the small bowel and colon in patients with scleroderma
[5]. These sacculations
represent true diverticula and are thought to result from abnormal intestinal
motility with outward ballooning of the bowel wall between asymmetric areas of
smooth muscle fibrosis and atrophy
[1,
5]. We therefore, postulate
that the wide-mouthed sacculations in the esophagus (Fig.
1A,1B)
in our patient had a similar pathophysiologic basis, resulting from abnormal
motility and asymmetric fibrosis and atrophy in the smooth muscle layer of the
esophageal wall.
One of the wide-mouthed sacculations in our patient was located in the
upper thoracic esophagus above the aortic arch. This location is a potential
concern because the transition zone between striated and smooth muscle in the
esophagus classically occurs at the level of the aortic arch
[2], and these sacculations
would not be expected to develop in regions of striated muscle. However,
postmortem studies of the esophagus have shown that this transition zone is
quite variable, sometimes occurring as far proximally as the thoracic inlet or
even the cervical esophagus [6,
7]. In our patient, there was
absence of peristaltic contractions in the entire thoracic esophagus on
manometry and absence of primary peristalsis in the thoracic esophagus above
and below the level of the aortic arch on fluoroscopy. These findings
suggested that the transition zone between striated and smooth muscle was
located above the aortic arch in our patient, presumably explaining the
development of the more proximal sacculation.
The wide-mouthed sacculations in the esophagus in our patient with
scleroderma must be differentiated from pulsion or traction diverticula.
However, pulsion diverticula tend to be deeper rounder outpouchings with
narrow necks and often are associated with multiple nonperistaltic
contractions rather than with a flaccid aperistaltic esophagus
[8]. In contrast, traction
diverticula tend to have a more tented angulated contour and frequently are
located at the level of the pulmonary hila as a result of scarring from prior
granulomatous disease in this region
[8]. Finally, peptic strictures
occasionally are associated with one or more sacculations caused by outward
ballooning of the esophageal wall between areas of fibrosis
[3]. However, these
sacculations are almost always located in the distal esophagus in the region
of the underlying stricture. Thus, it should be possible to distinguish the
wide-mouthed sacculations in scleroderma from other types of outpouchings in
the esophagus.
In summary, we have reported an unusual case of wide-mouthed sacculations
or diverticula in the esophagus similar to those in the small bowel or colon
in patients with scleroderma. Despite its rarity, we believe this finding
should be highly suggestive of esophageal involvement by scleroderma,
particularly if associated with a dilated aperistaltic esophagus and free
gastroesophageal reflux on fluoroscopy.
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